Subcutaneous Emphysema, Pneumomediastinum and Pneumothorax in a Patient with Dermatomyositis

Document Type: Case Report

Authors

1 Sinus and Surgical Endoscopic Research Center, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.

2 Department of Otorhinolaryngology-Head & Neck Surgery, Mashhad University of Medical Sciences, Mashhad, Iran.

Abstract

Introduction:
Spontaneous pneumomediastinum, pneumothorax, and subcutaneous emphysema are rare, but serious complications of inflammatory myopathies and occur more commonly in DM than PM. complications of dermatomyositis (DM) and polymyositis (PM), both of which can be fatal.
 Case Report:
A 20-year-old woman was admitted with neck pain, dyspnea, cough, and fever. She had been diagnosed with dermatomyositis 21 months prior. A thorax computed tomography (CT) scan revealed ground glass opacities in her lungs, pneumomediastinum, pneumothorax, and subcutaneous emphysema. Despite intensive immunosuppressive therapy, clinical deterioration and radiological progression were observed, ultimately the patient died.
 Conclusion:
During the care for a patient with dermatomyositis, the otorhinolaryngologist should be cautious of rapidly progressive and fatal neck subcutaneous emphysema. For a patient with dermatomyositis and with normal bronchoscopy and esophagoscopy, the main treatment is control of dermatomyositis with medical therapy. Therefore, a tracheostomy and/or mechanical ventilation may not be necessary.
 

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1. Connors GR, Christopher-Stine L, Oddis CV, Danoff SK. Interstitial lung disease associated with the idiopathic inflammatory myopathies: what progress has been made in the past 35 years? Chest 2010; 138 (6):1464-74.

2. Le Goff B, Chérin P, Cantagrel A, Gayraud M, Hachulla E, Laborde F, et al. Pneumomediastinum in interstitial lung disease associated with dermatomyositis and polymyositis. Arthritis Rheum 2009; 61(1):108-18.

3.Kono H, Inokuma S, Nakayama H, Suzuki M. Pneumomediastinum in dermatomyositis: association with cutaneous vasculopathy. Ann Rheum Dis 2000; 59(5):372-6.

4. Marie I, Hachulla E, Cherin P, Dominique S, Hatron PY, Hellot MF, et al. Interstitial lung disease in polymyositis and dermatomyositis. Arthritis Rheum 2002; 47: 614–22.

5. Rodrigues AJ, Jacomelli M, Scordamaglio PR, Figueiredo VR. Spontaneous pneumomediastinum associated with laryngeal lesions and tracheal ulcer in dermatomyositis. Rev Bras Reumatol 2012; 52(5):  796-9.

6. John Bradley. Spontaneous pneumomediastinum in adult Dermatomyositis. Annals of the Rheumatic Diseases. 1986; 45: 780-2.

7.Kono H, Inokuma S, Nakayama H, Suzuki M. Pneumomediastinum in dermatomyositis association with cutaneous vasculopathy. Ann Rheum Dis. 2000; 59: 372–6.

8. Ye S, Chen XX,  Lu XY, Wu MF, Deng Y, Huang WQ, et al. Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort study. ClinRheumatol 2007; 26: 1647–54.

9.Matsuda Y, Tomii M, Kashiwazaki S. Fatal Pneumomediastinumin Dermatomyositis without creatin kinase elevation. internal Medicine. 1993; 32: 643-7.

10.Gürün Kaya A, ÇiledaL A, KüçükGahin O, ÖzdemirKumbasar Ö, Atasoy Ç. A Case of Amyopathic Dermatomyositis with Pneumomediastinum and Subcutaneous Emphysema. Case Reports in Rheumatology. 2015; Article ID 813902.

11. Neves Fde S, Shinjo SK, Carvalho JF, Levy-Neto M, Borges CT. Spontaneous pneumomediastinum and dermatomyositis may be a not so rare association: report of a case and review of the literature. ClinRheumatol 2007; 26: 105-7.

12.Cozzani E, Cinotti E, Felletti R, Pelucco D, Rebora A, Parodi A. Amyopathic dermatomyositis with lung involvement responsive to mycophenolate mofetil. Immunopharmacol Immunotoxicol. 2013;35(6):
687-92.